نتایج جستجو برای: familial mds

تعداد نتایج: 63422  

Journal: :Blood 2014
Michael A Spinner Lauren A Sanchez Amy P Hsu Pamela A Shaw Christa S Zerbe Katherine R Calvo Diane C Arthur Wenjuan Gu Christine M Gould Carmen C Brewer Edward W Cowen Alexandra F Freeman Kenneth N Olivier Gulbu Uzel Adrian M Zelazny Janine R Daub Christine D Spalding Reginald J Claypool Neelam K Giri Blanche P Alter Emily M Mace Jordan S Orange Jennifer Cuellar-Rodriguez Dennis D Hickstein Steven M Holland

Haploinsufficiency of the hematopoietic transcription factor GATA2 underlies monocytopenia and mycobacterial infections; dendritic cell, monocyte, B, and natural killer (NK) lymphoid deficiency; familial myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML); and Emberger syndrome (primary lymphedema with MDS). A comprehensive examination of the clinical features of GATA2 deficiency is cu...

Journal: :Blood 2008
Carolyn J Owen Cynthia L Toze Anna Koochin Donna L Forrest Clayton A Smith Jane M Stevens Shannon C Jackson Man-Chiu Poon Gary D Sinclair Brian Leber Peter R E Johnson Anthony Macheta John A L Yin Michael J Barnett T Andrew Lister Jude Fitzgibbon

Familial platelet disorder with propensity to myeloid malignancy (FPD/AML) is an autosomal dominant syndrome characterized by platelet abnormalities and a predisposition to myelodysplasia (MDS) and/or acute myeloid leukemia (AML). The disorder, caused by inherited mutations in RUNX1, is uncommon with only 14 pedigrees reported. We screened 10 families with a history of more than one first degre...

Journal: :Blood 2013
Marlène Pasquet Christine Bellanné-Chantelot Suzanne Tavitian Naïs Prade Blandine Beaupain Olivier Larochelle Arnaud Petit Pierre Rohrlich Christophe Ferrand Eric Van Den Neste Hélène A Poirel Thierry Lamy Marie Ouachée-Chardin Véronique Mansat-De Mas Jill Corre Christian Récher Geneviève Plat Françoise Bachelerie Jean Donadieu Eric Delabesse

UNLABELLED Congenital neutropenia is a group of genetic disorders that involve chronic neutropenia and susceptibility to infections. These neutropenias may be isolated or associated with immunologic defects or extra-hematopoietic manifestations. Complications may occur as infectious diseases, but also less frequently as myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Recently, t...

Journal: :Blood 2012
Norio Shiba Daisuke Hasegawa Myoung-ja Park Chisato Murata Aiko Sato-Otsubo Chitose Ogawa Atsushi Manabe Hirokazu Arakawa Seishi Ogawa Yasuhide Hayashi

Familial platelet disorder with a propensity to develop acute myeloid leukemia (FPD/AML) is a rare autosomal dominant disease characterized by thrombocytopenia, abnormal platelet function, and a propensity to develop myelodysplastic syndrome (MDS) and AML. So far, > 20 affected families have been reported. Recently, a second RUNX1 alteration has been reported; however, no additional molecular a...

2015
Emmanuel Gyan François Dreyfus Pierre Fenaux

The 2008 WHO classification identified refractory cytopenia with unilineage dysplasia (RCUD) as a composite entity encompassing refractory anemia, refractory thrombocytopenia (RT), and refractory neutropenia (RN), characterized by 10% or more dysplastic cells in the bone marrow respective lineage. The diagnosis of RT and RN is complicated by several factors. Diagnosing RT first requires exclusi...

2015
Muhammad A Mir Samith T Kochuparambil Roshini S Abraham Vilmarie Rodriguez Matthew Howard Amy P Hsu Amie E Jackson Steven M Holland Mrinal M Patnaik

Guanine-adenine-thymine-adenine 2 (GATA2) mutated disorders include the recently described MonoMAC syndrome (Monocytopenia and Mycobacterium avium complex infections), DCML (dendritic cell, monocyte, and lymphocyte deficiency), familial MDS/AML (myelodysplastic syndrome/acute myeloid leukemia) (myeloid neoplasms), congenital neutropenia, congenital lymphedema (Emberger's syndrome), sensorineura...

Journal: :Blood 2003
Hironori Harada Yuka Harada Hideo Tanaka Akiro Kimura Toshiya Inaba

Somatically acquired point mutations of AML1/RUNX1 gene have been recently identified in rare cases of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). Moreover, germ line mutations of AML1 were found in an autosomal dominant disease, familial platelet disorder with predisposition to AML (FPD/AML), suggesting that AML1 mutants, as well as AML1 chimeras, contribute to the transfo...

پایان نامه :وزارت علوم، تحقیقات و فناوری - دانشگاه گیلان - دانشکده علوم ریاضی 1393

در این پایان نامه، ابتدا مفاهیمی مانند بازگشتی، ارگودیک، مولفه های تجزیه نا پذیر، فاکتور، آمیختگی را تعریف می کنیم و خواص آنها را در سیستمهای دینامیکی اندازه ای ‎(mds)‎ و سیستمهای دینامیکی توپولوژیکی tds)‎) تخمین می زنیم. همچنین نقش متقابل از این خواص را در ‎(mds)‎ و ‎(tds)‎ مورد مطالعه قرار می دهیم.

Myelodysplastic syndromes (MDSs) are a clonal bone marrow (BM) disease characterized by ineffective hematopoiesis, dysplastic maturation and progression to acute myeloid leukemia (AML). Methylation silencing of HRK has been found in several human malignancies. In this study, we explored the association of HRK methylation status with its expression, clinical parameters and MDS subtypes in MDS pa...

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